POS0939 PLEUROPARENCHYMAL FIBROELASTOSIS: A SPECIAL CLINICAL SITUATION IN PATIENTS WITH INTERSTITIAL LUNG DISEASE ASSOCIATED WITH CONNECTIVE TISSUE DISEASES. DESCRIPTIVE STUDY FROM A REFERRAL CENTRE
Authors: B. Atienza-Mateo, S. Remuzgo Martinez, V. Pulito-Cueto, D. Ferrer, G. Blanco, S. Izquierdo, V. M. Mora-Cuesta, D. Iturbe Fernández, R. López-Mejías, R. Blanco, J. M. Cifrián-Martínez, M. A. González-Gay
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease (ILD) that can be idiopathic or associated with a variety of different conditions, including connective tissue diseases (CTD) <b>[1-2]</b>. In this regard, the presence of PPFE has been reported as an independent predictor of worse prognosis in CTD-ILD patients <b>[3]</b>. Approximately one third of the patients with ILD meet criteria for a CTD <b>[4]</b>. <h3>Objectives</h3> <b>A</b>) To determine the prevalence of PPFE in a cohort of Spanish patients with CTD-ILD, and <b>B</b>) to compare the characteristics between CTD-ILD patients with and without PPFE. <h3>Methods</h3> A total of 99 patients with CTD-ILD (31 rheumatoid arthritis (RA), 31 systemic sclerosis (SSc), 21 idiopathic inflammatory myositis (IIM), 6 primary Sjögren's syndrome (SS), 4 systemic lupus erythematosus (SLE) and 6 with other CTDs) from the Hospital Universitario Marqués de Valdecilla (Santander, Spain) were included in this study. The presence of PPFE was confirmed by experienced radiologists evaluating chest high-resolution computed tomography images from all patients. In addition, demographic, clinical and radiological characteristics were collected. <h3>Results</h3> The presence of PPFE was found in 9 (9.1%) of the 99 CTD-ILD patients, whereas the remaining 90.9% had no signs of PPFE. In particular, it was confirmed in 4 patients with RA (12.9%), 2 with SSc (6.5%), 1 with IIM (4.8%), 1 with primary SS (16.7%) and 1 with SLE (25.0%) (<b>Figure 1</b>). The prevalence of bronchial dilation was statistically higher in CTD-ILD patients with PPFE compared to those without PPFE (44.4% <i>versus</i> 6.74%, respectively, p=0.006, <b>Table 1</b>). There were no significant differences in age at CTD or ILD diagnosis, sex, smoking history, body mass index and pulmonary function tests at ILD diagnosis between both groups (<b>Table 1</b>). <h3>Conclusion</h3> We provide the prevalence and clinical data of PPFE in patients with CTD-ILD from a national referral centre. Approximately 1 out of 10 of our patients presented associated PPFE, closely correlated to bronchial dilation. The identification of this condition in CTD-ILD patients should be considered routinely since it may worsen their prognosis. <h3>References</h3> [1]Chua F, et al. Ann Am Thorac Soc. 2019 Nov;16(11):1351-1359. doi: 10.1513/AnnalsATS.201902-181CME [2]Bonifazi M, et al. Eur Respir J. 2020 Jul 9;56(1):1902135. doi: 10.1183/13993003.02135-2019 [3]Orlandi M, et al. Rheumatology (Oxford). 2020 Dec 1;59(12):3645-3656. doi: 10.1093/rheumatology/keaa451 [4]Atienza-Mateo B, et al. J Clin Med. 2020 May 26;9(6):1606. doi: 10.3390/jcm9061606 <h3>Acknowledgements</h3> VP-C is supported by funds of PI18/00042 from Instituto de Salud Carlos III (ISCIII), co-funded by European Regional Development Fund; RL-M is a recipient of a Miguel Servet type II Program fellowship from ISCIII, co-funded by the European Social Fund, `Investing in your future ́ (CPII21/00004). <h3>Disclosure of Interests</h3> Belén Atienza-Mateo: None declared, Sara Remuzgo Martinez: None declared, Verónica Pulito-Cueto: None declared, Diego Ferrer: None declared, Gerardo Blanco: None declared, Sheila Izquierdo: None declared, Victor Manuel Mora-Cuesta: None declared, David Iturbe Fernández: None declared, Raquel López-Mejías: None declared, Ricardo Blanco Speakers bureau: Abbvie, Pfizer, Roche, Lilly, Bristol-Myers, Janssen, Galapagos and MSD, Consultant of: Abbvie, Pfizer, Roche, Lilly, Bristol-Myers, Janssen and MSD, Grant/research support from: Abbvie, MSD, Novartis and Roche, Jose Manuel Cifrián-Martínez: None declared, Miguel A González-Gay Speakers bureau: Abbvie, Pfizer, Roche, Sanofi, Lilly, Celgene, MSD and GSK, Grant/research support from: Abbvie, MSD, Jansen and Roche.